Sickle Cell Disease has been an area of high priority for the National Institute for Health Research Collaboration for Leadership in Applied Health Research and Care Northwest London (NIHR CLAHRC NWL) for many years. CLAHRC NWL has funded a multitude of sickle cell disease related projects for the past seven years ranging from ‘My Medication Passport’ to the Sickle Cell Disease patient reported experience measure (PREM).

Sickle Cell PREM

The Sickle Cell PREM was the first nationwide survey of people living with Sickle Cell Disease (SCD). It was designed by the Picker Institute, the not-for-profit charity that uses people’s experiences to improve the quality of health and social care for all. NIHR CLAHRC NWL, in partnership with the Sickle Cell Society, commissioned the development and implementation of the questionnaire.

The focus of the survey was to capture the true essence of patient experience, asking patients about what aspects of their care is important to them. The survey concentrated on access to care services, information and support for their condition, as well as capturing views on care across inpatient, outpatient and emergency care settings.

The survey had different design formats to suit the target audience of paediatric and adult patients, and parents/ carers of young SCD patients. It successfully received over 720 responses, with a minimum of 200 for each target audience. It is the largest exercise, conducted in the UK to date for obtaining feedback from people living with the condition.

Having the survey specifically tailored to the needs of the sickle cell patient group will enable the ability to effectively capture and improve their care experiences as well as potentially informing the delivery, design and commissioning of services.

Speaking on the success of the survey, John James, Chief Executive of the Sickle Cell Society said; “We have been overwhelmed by the sheer generosity of experience shown by the sickle cell community, and firstly just want to say a massive thank you to them and everyone that has worked so hard to generate responses. In the past we have heard of extremely varied care experiences from people with sickle cell, from truly inspiring exemplary care, to, what can only be described as disturbing. The new survey gives us the ability to measure experiences using a validated tool, and will allow us to obtain a wealth of data on sickle cell care, which will be a real game-changer in terms of the care improvements it could enable.

As well as inspiring grant funding for further research, the SCD survey tool will be made available for individual clinicians in England to collate relevant feedback and make service and care quality changes accordingly. Your feedback is invaluable and will make a real difference; we look forward to updating you on just how it is being used soon.”

Final data is currently being analysed by the Picker Institute and the key findings will be shared as soon as they are available. For future updates on the sickle cell PREM please subscribe by clicking the ‘Follow CLAHRC NWL’ button in the sidebar!

CLAHRC NWL Sickle Cell Improvement Projects

CLAHRC NWL currently has a number of other sickle cell improvement projects that have been developed over the last year. These projects are:

Sickle Cell Services in Brent – Sickle Cell Society has been commissioned to provide a SCD service that GPs can refer to in Brent.

This Sickle Cell Life: Sickle Cell Disease Transition to Adult Services – A study on young people’s experiences of transitioning from paediatric to adult services and about young people’s experiences of living with sickle cell. Currently recruiting.

Sickle Cell Disease is a genetic chronic blood disorder that affects the red blood cells. The red blood cells contain a protein called haemoglobin (Hb). The fundamental role of Hb is to carry oxygen around the body. Typically red blood cells have haemoglobin A (HbA) whereas for those who have sickle cell, their red blood cells have haemoglobin S (HbS). Red blood cells are usually round (disc-shaped) and flexible allowing the cells to easily bend and flex to travel through the blood vessels. Those who have sickle cell disease have abnormal red blood cells that become sickle (banana) shaped, ridged and have a shorter lifespan meaning that it breaks down at a faster rate than regular red blood cells.

The cells shape makes it difficult for them to pass through the blood vessels leading to blockages. The blockages make it difficult for oxygen to travel freely around the body resulting in excruciating pain as well as potential tissue and organ damage. There are many other serious and life threatening complications that are associated with sickle cell disease such as:

Anaemia

Aplastic Crisis

Vulnerability to Infection

Jaundice and Gallstones

Avascular necrosis

Transcranial Ischemic Attacks also known as TIAs and Min-Strokes

Priapism

Although there is the potential risk for somebody with sickle cell to receive some of the above complications, as well as others unmentioned, it is not definitive. The uniqueness of each individual with sickle cell disease and how they are affected varies from case to case and the journey of medical implications for any individual can change at any time.

Photo Credit: The National Heart, Lung, and Blood Institute (NHLBI)Photo Credit: The National Heart, Lung, and Blood Institute (NHLBI)

Demographics and Statistics

Sickle Cell mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. However cases of this disease can be found across all backgrounds. According to NHS UK, Sickle cell disorder affects between 12,500 and 15,000 people in the UK, making it the most common inherited disease in the country.

How do you get Sickle Cell?

Sickle cell is an inherited blood disorder that is passed down from the parents. Every baby born in the UK is screened for the condition. In order for a person to have sickle cell disease, both parents need to either be a carrier of the sickle cell gene or have the condition themselves. If a person is a carrier of the gene they are classed as having sickle cell trait. When both parents have the trait there is a 1 in 4 chance with each conception that their child will be born with sickle cell disease, a 2 in 4 chance that their child will also be a carrier and a 1 in 4 chance that their child will not have any trace of sickle cell disease . There are many variations in which a person could inherit the condition, which can be seen in the diagram below. (Editor’s Note: Please be aware that the diagram below does not validate gender specific inheritance, sickle cell inheritance has no relation to the gender of the child.)

Image Retrieved from The Free Medical Dictionary. Origins Unknown.

Quality of Life

Sickle Cell Disease can affect an individual spontaneously at any stage of their life. There are certain triggers that can increase the likelihood of a sickle cell crisis such as; stress, dehydration, weather conditions and over exertion. People with sickle cell disease tend to have a low immune system, making them more susceptible to other illnesses and viruses. Due to this, it is a common recommendation from haematology consultants to prescribe Folic Acid, to help produce and maintain new cells, and Penicillin, to reduce the risk of infection. It is not uncommon for somebody with sickle cell to be well one moment and extremely unwell the next. This means that sickle cell can affect students in school, college and university and can have an impact on work and various social activities. Sickle Cell Disease is covered by the Children and Families Act 2014, the Equalities Act 2010 as well as the statutory guidance for Supporting Pupils at School with Medical Conditions.

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A Powerful Message

Editors Note: Contact information at the end of the videos is not relevant for the UK public. If you have questions or want to find our more, please contact your local GP for advice. Alternatively seek help and information from your local sickle cell centre, local support group or the national charity Sickle Cell Society.